Journal
NEUROREPORT
Volume 11, Issue 6, Pages 1361-1364Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/00001756-200004270-00042
Keywords
basal ganglia; catecholamines; mutant mice; phenylalanine hydroxylase; phenylketonuria; serotonin; tyrosine; tryptophan
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Clinical data suggest that brain catecholamines and serotonin are deficient in phenylketonuria (PKU), an inherited metabolic disorder that causes severe mental retardation and neurological disturbances. To test this hypothesis, brain tissue levels of dopamine (DA), norepinephrine (NE), 5-hydroxytryptamine (5-HT) and their metabolites were evaluated in the genetic mouse model of PKU (Pah(enu2)). Results indicated a significant reduction of 5-HT levels and metabolism in prefrontal cortex (pFC), cingulate cortex (Cg), nucleus accumbens (NAc), caudate putamen (CP), hippocampus (HIP) and amygdala (AMY). NE content and metabolism were reduced in pFC, Cg, AMY and HIP. Finally, significantly reduced DA content and metabolism was observed in pFC, NAc, CP and AMY. In pFC, NAc and CP there was also a marked reduction of DA release. NeuroReport 11:1361-1364 (C) 2000 Lippincott Williams & Wilkins.
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